Background: To determine the demographics of the first 1,000 families in the Gilda Radner Familial Ovarian Cancer Registry.
Methods: Any woman with a family history of two or more first or second degree relatives with ovarian cancer who was referred to the Registry was entered into a database. The demographics of the Registry population were analyzed, including total number of ovarian cancer cases, cases per family, age at diagnosis, prevalence of other malignancies, and histology. Comparisons were made to expected norms in the general population using the most recent surveillance, Epidemiology, and End Results data from the National Cancer Institute.
Results: There were 2,425 reported cases of ovarian cancer among the 1,000 registered families. The number of cases per family ranged from 2-10 with 72.4% of the families having two cases of ovarian cancer and 27.6% with three or more cases of ovarian cancer. The mean age at diagnosis was 53.5 years, significantly younger than the 60.8 years reported in the general population (p < 0.1). The mean age at diagnosis was even younger, 52.1 years, in the subset of patients with a family history of three or more cases of ovarian cancer. Moreover, in the mother/daughter pairs of ovarian cancer where age of onset of ovarian cancer was known, the mean age of the mothers was 58.8 compared to a mean age of 50.2 among the daughters (p < 0.01). There was a significantly larger proportion of serous adenocarcinoma (40%) and a significantly smaller proportion of mucinous adenocarcinoma (3%) and stromal cell tumor (0%) when compared to the general population (p < 0.01). Breast and colon cancer were the two most common other cancers reported.
Conclusions: given the limits of the study due to the voluntary nature of the Registry, the data suggests that familial ovarian cancer occurs at a younger age than the general population, daughters of mothers with ovarian cancers develop ovarian cancer at an age significantly younger than their mothers, mucinous and stromal tumors occurred significantly less often than in the general population, and the most common associated other cancers are female breast cancer and female and male colon cancer.